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July 13, 1964


JAMA. 1964;189(2):149. doi:10.1001/jama.1964.03070020077020

Metabolic defects are being noted more frequently as prominent components of various hereditary or congenital syndromes. In some cases the metabolic aberrations account for many of the clinical findings; in other instances it is not yet possible to prove that they play a causative role. An example of the latter type is found in a report in the July issue of the Archives of Dermatology.1

Clendenning et al studied a group of six patients with the basal-cell nevus syndrome. The components of this syndrome are primarily basal-cell epitheliomas of the skin, with various bone defects such as mandibular cysts and minor rib anomalies. Other abnormalities may involve the soft tissues (calcification, ovarian fibromas, or lymphatic mesenteric cysts) or the nervous system (mental retardation, nonspecific electroencephalographic changes, various abiotrophics ). The inheritance pattern is dominant, but with variable expressivity.2 Some of the defects suggest a relationship between the syndrome and

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